Objective:To analyze the efficacy of enzyme replacement therapy and anti-drug antibody production in children with Fabry disease.Methods:The clinical data of 7 children with Fabry disease treated with enzyme replacement therapy for more than 1 year at Children′s Hospital of Zhejiang University School of Medicine from July 2021 to June 2024 were retrospectively analyzed. The basic information and the changes of related clinical indicators before and after treatment were collected. Paired sample t test was used to compare renal function, left heart mass index, pain score and other related indexes before and after treatment. The anti-drug antibodies were detected by enzyme-linked immunosorbent assay. Results:A total of 6 boys and 1 girl were included. The age of diagnosis was (12.2±1.8) years. After 1 year of enzyme replacement therapy, the abnormal substrate globotriaosylsphingosine and brief pain inventory scores of all children were significantly lower than those before treatment ((16±11) vs. (63±42) μg/L, 22±19 vs. 45±29, t=3.88, 3.43, both P<0.05). There were no significant differences in glomerular filtration rate, urinary microalbumin to creatinine and left heart mass index before and after treatment ((124±35) vs. (136±26) ml/(min·1.73 m 2), (9.3±8.3) vs. (3.8±2.5) mg/g, (38±9) vs. (33±6) g/m 2.7, t=1.33, 1.74, 1.19, all P>0.05). Patients 4, 5 and 6 developed anti-drug antibodies at 1 month, 4 months and 1 month after medication, respectively. Patient 4 had persistently high anti-drug antibody titers (absorbance 3.65-3.73) accompanied by urticaria, elevated globotriaosylsphingosine and worsening clinical symptoms. Conclusions:The enzyme replacement therapy can effectively improve the clinical symptoms and reduce the level of globotriaosylsphingosine in children with Fabry disease. The anti-drug antibody is common in patients after long-term enzyme replacement therapy and may diminish the efficacy, which needs dynamic monitoring.

ObjectiveTo observe the efficacy and safety of Uyghur medicine Yangxin Dawayimixike Honey Paste （养心达瓦依米西克蜜膏， YDMHP） in the treatment of stable angina pectoris （SAP） of qi stagnation and blood stasis syndrome. MethodsA randomized ， double-blind， positive-controlled，multi-center clinical trial was conducted， in which 370 patients with SAP of qi stagnation and blood stasis syndrome were randomly divided into treatment group（279 cases）and control group（91cases）at a ratio of 3∶1. The treatment group was orally administered with YDMHP， 3 g each time， and placebo of Xuefu Zhuyu Capsule （血府逐瘀胶囊）， 2.4 g each time， while the control group was treated with Xuefu Zhuyu Capsule， 2.4 g each time， and placebo of YDMHP， 3 g each time， both twice a day for a course of 12 weeks. The primary outcome was the effect of angina pectoris symptom. The secondary outcomes include single angina symptom scores such as number of attacks， duration of attacks， pain intensity and usae of nitroglycerin scores， the total angina symptom score before and after the treatment， the usage of nitroglycerin， the exercise duration in treadmill exercise test （TET） and the Duck treadmill score among patients，the scores of Seattle Angina Questionnaire （SAQ） on five dimensions including physical limitations， anginal stability， anginal frequency， treatment satisfaction， and disease perception， and efficacy of TCM syndrome and of each single TCM symptom after treatment. The safety were evaluated by examine blood routine， urine routine， liver and kidney function， fasting blood sugar， electrocardiogram， adverse events. ResultsThe total effective rate of angina symptom in the treatment group was 71.69% （200/279）， significantly higher than 51.64% （47/91） in the control group （P＜0.01）. The curative and markedly effective rate of TCM syndrome in the treatment group was 53.05% （148/279）， which was significantly higher than 25.27% （23/91） in the control group （P＜0.01）. After treatment， scores of the number as well as duration of angina attacks and pain severity， the total score of angina symptoms， and the usage of nitroglycerin significantly decreased in both groups， and more changes were seen in the treatment group than in the control group； the scores of physical limitations， anginal stability， anginal frequency， treatment satisfaction， and disease perception in both groups significantly increased， and more improvement were shown in the experimental group regarding the anginal stability， anginal frequency and treatment satisfaction （P＜0.05 or P＜0.01）. The effects of chest pain， chest tightness， palpitation， shortness of breath and fatigue in experimental group were significantly higher than those in control group （P＜0.05 or P＜0.01）. There was no significant difference in the exercise duration of treadmill test and Duke score among patients between the two groups either before or after treatment （P＞0.05）. Adverse events occurred in 66 cases （23.66%） of the experimental group and 16 cases （17.58%） of the control group， with no statistical significance between the two groups （P＞0.05）. ConclusionThe Uyghur medicine YDMHP can effectively improve symptoms of angina pectoris， reduce the number， duration， and intensity of attacks， decrease the dosage of nitrogly-cerin and improve the individual TCM symptoms and has good safety in the treatment of SAP patients of qi stagnation and blood stasis.

【Objective】 To assess the prevalence of chronic diseases and associated influencing factors among residents in Ali so as to provide reference for local prevention and control of chronic diseases. 【Methods】 Stratified cluster random sampling was used to investigate the prevalence of chronic diseases and their influencing factors among permanent residents over 18 years of age in Aili. 【Results】 Totally 606 subjects were included, with a prevalence of chronic diseases of 39.93% and the highest prevalence of rheumatoid arthritis of 14.03%. The prevalence of chronic diseases was significantly different among ethnicity, age, education, occupation, personnel category, family living standard, amateur physical exercise, fuel type and self-rated health (P<0.05). Logistic regression showed that the risk of chronic diseases among the residents aged 50 years and above was 2.656 (95% CI: 1.439-4.901) times higher than that of residents under 30 years; the risk of chronic diseases among the residents with elementary school education and below was 3.528 (95% CI: 1.519-8.197) times higher than that of residents with college education and above; housework personnel could reduce the risk of chronic diseases (OR=0.365, 95% CI: 0.161-0.828); residents with poor self-rated health had 5.814 (95% CI: 3.243-10.423) times higher risk of chronic diseases than those with good self-rated health; and those with a weight gain of more than 2.5 kg in the past year could increase the risk of chronic diseases (OR=2.029, 95% CI: 1.226-3.358). 【Conclusion】 The prevalence of chronic diseases is high in residents of Ali. Attention should be paid to the elderly population with poor self-rated health and tendency of weight gain. Personalized behavioral lifestyle health education is helpful in preventing the occurrence of chronic diseases.

Objective:To explore the mediating effect of caring ability of family caregivers on dementia patients′ self-care ability and family caregiver burden.Methods:A total of 113 dementia caregivers of dementia patients recruited in Shandong Provincial Hospital Affiliated to Shandong First Medical University and Shandong Mental Health Center from September 2020 to January 2021 were selected, and the Barthel Index, Family Caregiver Task Inventory and Zarit Burden Interview were used to capture their responses on dementia patients′ self-care ability, caring ability, and caregiver burden.Results:The score of self-care ability of dementia patients was (75.19 ± 28.56), and scores of caring ability and care burden of caregivers were 9.00(4.00, 15.00) and (33.78 ± 16.53), respectively. The self-care ability of dementia patients was negatively associated with caring ability and care burden of caregivers ( r=-0.424, -0.420, both P<0.01), and the caring ability and care burden were positively correlated ( r=0.605, P<0.01). The intermediary effect of dementia caregivers' caring ability between patients' self-care ability and caregivers' care burden is significant. The indirect effect was -0.107 and the total effect was -0.187. The indirect effect accounted for 57.22% of the total effect. Conclusions:Caring ability of family caregivers mediates the relationship between self-care ability of dementia patients and caregivers' care burden.The caring ability may be used as an intervening target for future studies.

In this study, a Specific Pathogen-Free (SPF) Bama miniature pig herd was established.The standards for breeding experimental pigs, genetic test and microbiological quality control had been drafted.The local standards of Heilongjiang province SPF technical specifications for microbiological monitoring of pigs had been formulated.The genetic and microbiological quality criterion had been used to control of the SPF pig herd.Classical swine fever virus (CSFV) and highly pathogenic porcine reproductive and respiratory syndrome virus (PRRSV) infection models of Bama miniature pigs were established, especially the infection miniature pig model of PRRSV was used to evaluated commercially vaccine.The comparison of the cytokine homology between Bama miniature pigs and domestic pigs showed that the Bama miniature pigs can be used to instead of domestic pigs as an ideal experiental animal.At present the SPF Bama miniature pig colonies have been widely used to study the mechanism of prevention and pathogenic in the classical swine fever virus, porcine reproductive and respiratory syndrome virus and porcine transmissible gastroenteritis virus and porcine circovirus and so on.The completion of the project solved the bottleneck problem of the experimental pig usage which provided a new ideal experimental animal for animal disease and life science research.

Objective To investigate the treatment effect of ginsenoside compound K (CK) on glucose and lipid metabolism in diabetic mellitus mice and the potential molecular mechanism.Methods A total of 36 mice were randomly divided into normal group,diabetic mellitus group,CK treatment groups (100 or 200 mg/kg body weight),dimethyldiguanide group and p38MAPK pathway agonist P79350 group,with 6 mice in each group.Diabetic mice were established by intraperitoneal injection of streptozotocin combined with high-fat diet,and CK with different doses was administrated by gastric lavage for consecutive 8 weeks.The levels of fasting blood-glucose,triglyceride (TG),total cholesterol (TC),high-density lipoprotein (HDL C),fasting serum insulin were measured,and the insulin sensitive index (ISI) was calculated in different treatment groups.Glucose tolerance was detected by oral glucose tolerance test.The protein levels of ASK1,p-ASK1 and p38,p-p38,was detected by Western blot.The mRNA expression of apoptosis signal regulating kinase-1 (ASK1) was detected by real-time quantitative PCR.Results The fasting blood-glucose,TG,TC,HDL C,fasting serum insulin and ISI were (28.31 ± 3.40),(1.90 ± 0.28),(5.00 ± 0.72),(0.50 ± 0.08),(9.01 ± 1.70) mmol/L and-6.42 ± 0.76 in diabetic mice,respectively.The corresponding values were (12.02± 1.81),(0.97 ±0.09),(2.90 ±0.49),(0.91 ±0.08),(15.12 ± 1.93)mmol/L and-4.33 ± 0.46 in 200 mg/kg CK treatment diabetic mice,and were (12.87 ± 2.61),(1.09 ± 0.11),(3.08 ± 0.27),(0.87 ±0.08),(14.97 ± 1.27) mmol/L and-4.42 ± 0.35 in dimethyldiguanide group.All of the fasting blood-glucose,TG and TC in CK treatment groups were significantly lower than those of diabetic mellitus group (P ＜0.05 or ＜0.01),but the fasting serum insulin and ISI in CK treatment groups were significantly higher than that of diabetic mellitus group (P ＜ 0.05 or ＜ 0.01).There were no significant difference between 200 mg/kg CK treatment group and dimethyldiguanide group.The mRNA levels of ASK1 in normal group,diabetic mellitus group and 200 mg/kg CK treatment group were 1.00 ± 0.07,2.52 ± 0.14 and 1.25 ± 0.08,respectively.The mRNA levels of ASK1 in diabetic mellitus group and 200 mg/kg CK treatment group were significantly up-regulated than that of normal group (P＜0.01),but there was no significant difference between 200 mg/kg CK treatment group and diabetic mellitus group in the mRNA levels of ASK1.There was no significant difference in the protein expression levels of ASK1 and p38 among normal group,diabetic mellitus group and 200 mg/kg CK treatment group,but the protein expression levels of p-ASK1 and p-p38 were significant higher in diabetic mellitus group than that in normal group (P＜0.05 or ＜0.01),and were significant lower in 200 mg/kg CK treatment group than that in diabetic mellitus group (P ＜ 0.05 or ＜ 0.01),and were no significant difference between 200 mg/kg CK treatment group and normal group.Conclusions Ginsenoside CK effectively attenuates diabetic mellitus in mouse model,possibly by inhibiting the phosphorylation of ASK1-p38MAPK signaling pathway.

Objective To explore the value of subglottic secretion drainage in the prevention of the late -onset ventilator associated pneumonia.Methods Clinical data of 273 patients which were treated for long -term mechanical ventilation and tracheotomy were retrospectively analyzed.Based on continued subglottic suction,the patients were divided into the subglottic suction group(132 cases)and control group(141 cases),the late -onset ventilator -associated pneumonia incidence of two groups were compared.Results The ventilator -associated pneu-monia rate of the subglottic suction group was 25.8%(34 /132),and the ventilator -associated pneumonia rate of the control group was 22.7%(32 /141 ),the difference between the two groups was not significant(χ2 =0.345,P >0.05).Conclusion In the prevention of late -onset ventilator -associated pneumonia,the effect of continuous sub-glottic suction is unclear.

Objective To explore the relationship between serum 25-hydroxy vitamin D level and prostatic hyperplasia in elderly men.Methods Totally 95 male patients aged over 60 years were included.Blood pressure,body weight,body height,body mass index (BMI) were measured and calculated.Venous blood samples were obtained to determine fasting serum levels of 25-hydroxy vitamin D3 and blood glucose (FBG),total cholesterol (TC),triglycerides (TG),low-density lipoprotein cholesterol (LDL-C),high-density lipoprotein cholesterol (HDL-C),calcium and prostate specific antigen (PSA),total prostate volume(PV) and annual prostate growth rate were measured and calculated by ultrasound.Results The serum 25 (OH) D3 levels were varied from 12.1 nmol/L to 83.9 nmol/L,with an average of (35.5±15.2) nmol/L in elderly male patients.PV growth rate were significantly lower in elderly men with 25 (OH) D3 ＞ 50 nmol/L than in elderly men with 25 (OH) D3≤50nmol/L[(31.5± 6.0) mlvs.(39.9 ± 14.5) ml,(0.4± 0.2) ml/yvs.(0.5 ± 0.4) ml/y,P＜0.001 or 0.01].PV was negatively correlated with serum 25-hydroxy vitamin D3 level (r=-0.207,P＜0.05),and positively correlated with BMI and PSA (r=0.297,0.958,P＜0.05 or and P＜0.001).While annual prostate growth rate was positively correlated with BMI and PSA (r=0.316,0.464,P＜0.01 or ＜0.001),and positively correlated with serum 25-hydroxy vitamin D3 level,but the difference was not statistically significant (P＞0.05).Conclusions Low serum 25-hydroxy vitamin D3 level may play a role in the pathogenesis of prostatic hyperplasia.

Objective To evaluate the relationship between insulin resistance and benign prostatic hyperplasia (BPH).Methods Totally 150 male patient from the Department of Geriatrics in Peking University Hospital were included in this study.Blood pressure,body weight,body height,body mass index (BMI) were measured and calculated.Biochemical analyses including serum fasting levels of insulin(FINS),glucose,total cholesterol,triglycerides,low-density lipoprotein cholesterol,high density lipoprotein cholesterol,and prostate-specific antigen (PSA) were performed.Total prostate volume (PV) were measured by ultrasound.Results PV and annual prostate growth rate were more increased in insulin resistance group(40 cases) compared with insulin sensitivity group(110 cases) (t=2.91,3.71 respectively,both P＜0.01).Along with the levels of FINS,HOMA-IR and PSA were increased,the prostate volume was enhanced (t=-3.02,-2.88,-2.84 respectively;all P ＜0.05).PV was positively correlated with insulin resistance,serum fasting insulin and PSA (r=0.16,0.16,0.35;all P＜0.05),while annual prostate growth rate was positively related with insulin resistance,serum fasting insulin,PSA and BMI (r =0.22,0.21,0.24,0.19 ; all P ＜ 0.05).Conclusions Insulin resistance and fasting insulin plays roles in the pathogenesis of prostatic hyperplasia.